Thursday, April 25, 2013

I have spent the past several months, if not the past year, working tirelessly to convince the CF Community at large that we need a Collaborative Chronic Care Network. One by one, slowly, I have somehow managed to either sufficiently educate or outright annoy enough people into listening that I've gotten some movement. It started in Cincinnati, using the C3N team and my position on the CF Quality Improvement team to show how processes could be made better, incomplete data filled in, patient engagement encouraged and outcomes improved. I got someone to listen, and then they got someone else to listen, and before I knew it we had a team (a small team but a team nonetheless) eager to find a way to make this happen. Then last fall, I made a phone call to the Cystic Fibrosis Foundation's national office to tell them about our idea. I got an admin who asked me to send an email with more information. And then I got a response that it would probably be some time before anyone had a chance to get back to me. That same day (that SAME DAY), she emailed me again saying that their VP of Clinical Affairs was intrigued and wanted to arrange a call. That's when I had to call Children's and tell them that I'd reached out and now we needed to get something together to share (it's easier to ask for forgiveness than for permission). I was delighted to hear that they were as excited as I was to engage with the CF Foundation and we worked together to present our project. I have to admit that looking back at that time, in Oct. 2012, I hadn't even fully grasped the project I was pitching, I just knew that it held potential and that I could continue to learn.

We didn't know it at that time [though I had a feeling], that call in October would be the beginning of a beautiful relationship. Since then, we were able to motivate more of the CF team in Cincinnati and even gain funding to move forward with several of the C3N intervention over the next year. The opportunity to collaborate with the CF Foundation would provide an incredible platform to move this forward, and we were starting to see that they felt the same way. Their VP of Clinical affairs came to Cincinnati in January and was very intrigued by the new way of managing healthcare that we were suggesting. Enough so that we continued our conversations over the next several weeks until we invited them back to talk more about how we could work together. That's when we found out that, not only would the one person who had come before be returning, but 7 members of their leadership team were interested in coming to Cincinnati to learn more. That meeting happened yesterday, it it was a good day. Like really, a really good day.

The group that came to listen to to our collaboration talks was nothing short of impressive. There were brilliant scientists, leadership from the Anderson Center at Cincinnati Children's, doctors, nurses, and a number of other collaborators. There were talks on what a C3N is as well as a number of other Learning Networks and how they are created, sustained and what makes them thrive. We talked about the business model of the CF Foundation and what we could learn from them in their venture philanthropy and collaborative efforts with pharmaceutical companies and others. There was so much amazing information sharing going on throughout the day that I knew that this partnership was meant to be.

The second half of our day was spent in breakout sessions sharing some of the different intervention platforms - Nof1 Learnings, an inter-visit planning tool, patient activation technologies - the list goes on and on. This was one of the most engaging parts of the day because people really got to see the application of our proposed interventions and how they could help the CF Community as a whole.

I had the opportunity throughout the day to share several conversations with Preston Campbell, the Senior VP of the CFF. I asked him a little bit about the new infection control guidelines for CFF events and what he saw as changing with the care center guidelines. One of the big reasons for the change to the guidelines for CFF events is that the benefit to the CFF (mostly our fundraising efforts to support them) was not worth the risk to patients. Not that they ever want to put patients at risk, but within the clinical setting, the benefit of seeing your care team on a regular basis outweighs that risk of catching a pathogen. It seems that there will be more gowning and gloving, less shared space, even more rigorous cleaning in between patients. He did mention that as these corrector drugs continue to develop, he sees the future of CF being one where a perfect combo is found for each and ever patient individually, not just grouped by mutation. Perhaps it will work out to be that certain classes of mutations find the most benefit from a particular combination of corrector drugs, but we will be able to use DNA swabs to test drug combos in a petri dish to see which will most effectively correct your disease. So cool.

The day wrapped up around 3pm with a round table attended by some absolutely incredibly brilliant people...and me. That's right, I had nothing to lose by sitting right up in the middle of the conversation adding my two cents on behalf of the rest of the CF patient/family community, so I did. Our hypothesis was validated - the CF Foundation called the work at Children's fascinating and innovative. They are interested in a collaboration of a capacity still to be determined, but a partnership nonetheless. This is so amazing for the CF community. This new system will allow for more individual health monitoring, self experimentation under the supervision of physicians, patient connection in a time when CF patients are being driven farther and farther apart by infection control scares, and overall improved outcomes via all of these new metrics. The benefits of a C3N for CF will be seen by patients, doctors, other clinicans, families, researchers, teachers...and the list goes on and on. I cannot wait to get started on this pilot project. Ultimately, and probably just in time for the last small percentage of the CF community to be "cured", we will have this new care system in place for everyone to benefit from. I'm gonna throw out a guess of 3-5 years, and its honestly just a guess (maybe an ambition). So get ready world, my goal of ultimate domination is slowly coming to fruition :)

Wednesday, April 24, 2013

The purpose of this message is to ask members of congress to sign on to a letter to the Social Security Administration being circulated by Congressman Ed Markey (D-MA) and Congressman Tom Marino (R-PA) that expresses concern with the proposed rule, "Revised Medical Criteria for Evaluating Respiratory System Disorders".

The "Revised Medical Criteria for Evaluating Respiratory System Disorders" proposal changes the medical criteria that the Social Security Administration uses to determine whether someone with cystic fibrosis is eligible for disability benefits, and physicians who specialize in CF say that these revisions do not accurately measure whether a person with cystic fibrosis is disabled. If left unchanged, the proposed rule could unfairly bar people with CF who cannot work from accessing these critical resources.

The most troubling provision of the proposed rule states that in the event of a serious complication, a CF patient would have to be hospitalized in order to show that they are becoming progressively sicker to the point of disability. However, doctors now have the ability to treat some very serious complications on an outpatient basis, and many doctors prefer this alternative due to the risk of hospital-acquired infection and the cost of hospital care. Requiring hospitalization goes against accepted medical practice and could impact CF patients' eligibility for benefits.

Once again, we are encouraging all members of congress to sign Representatives Markey and Marino's letter to the Social Security Administration (SSA) which expresses concern with the proposed "Revised Medical Criteria for Evaluating Respiratory System Disorders" rule and urges SSA to revise this proposal. For more information or to sign on, please contact Sara Schaumburg in Congressman Markey’s office at sara.schaumburg@mail.house.govor Drew Kent in Congressman Marino’s office at drew.kent@mail.house.gov.

To share this request with your member of congress and help to make a difference, click here:

My name is Erin Moore and I have a 3yr old son who has Cystic Fibrosis. This morning, I wanted to share with you a story about why I think that creating a Collaborative Chronic Care network for Cystic Fibrosis would be hugely beneficial to our community.

I arrive for our clinic appointment around 7:45am. The last time we were here was 3 months ago. Usually, we are ushered back to a room within 15 minutes of arrival. A nurse greets us shortly thereafter to review our medicine list and address any issues that we've been having. Just the other day he was coughing up a storm but seems to have gotten over it. Should I talk to the dietician about his diet again? His weight is up and his stool seem "normal" but I'm always anxious about his lack of interest in food. I wonder if an RT is available to talk a little bit about his airway clearance. I don't want to be a bother but I sure do think the airway clearance he had in the hospital this summer was more effective. I wish I knew what they were doing differently!

Next up is the doctor. She asks how things have been going. Today? Great! Last week? I was a little worried, but the cough he had seemed to go away. There was that one day that his stool was a little weird but that got better too, I’m not sure what caused it. And frankly I can't remember back farther than that. She checks him out and wants to review his labs since we are at his anniversary visit. His vitamin D is low, his breathing still sounds a little noisy, and a note that she has from his ENT seems to indicate that he may need another sinus surgery. She feels out whether I'd be open to a bronchoscopy at the same time. Maybe another CT is a better option. When I talked to the ENT last month it sounded like things were going well? I guess I don't mind if they do a bronchoscopy while he's under for something else, but I remember huge discussion with other CF parents on the Facebook about CT scans and all the negative effects of radiation and I don't know that I want to do that? I wish I could find that conversation! I ask questions about a game plan for if he needs IV antibiotics if we grow pseudomonas again, having read online about all different methods used for eradication but not knowing which is best and why. It's hypothetical at the moment because they haven't even swabbed him yet. I just have sort of a busy life and sometimes having a plan provides a sense of comfort for me. She suggests an action plan and I am mostly on board, except for the azithromycin because I saw a presentation somewhere that seemed to show compelling evidence against its use. I wish I could find that presentation to show her! I trust his doctor and want to follow her recommendations, but I have some reservations. I don't think either of us has time to get into this as I've already taken up more than my fair share of appointment time. We agree to wait for the results of the culture to decide a course of action.

Next up is the dietician. Lucky for me, he has a "weird poop" while we are there so she can look at it and provide her thoughts. This isn't what all of his stool looks like. I don't really know how often his stool looks like this - sometimes once a day, sometimes 4 times a day, sometimes once a week. We talk for a bit about enzyme dosing and the calories he's taking in, her offering suggestions for beefing up his intake and me adding them to the "notes" section in my iPhone, hoping I can get them down elsewhere before one of my kids deletes them by accident.

The social worker pops in to address some questions about starting preschool that I had mentioned to the nurse 2 hours ago. I had met with the Psychologist about a year ago to talk about his lack of interest in food, but never followed up, mostly because of both time constraints and cost. If only she could pop in and give me a couple of quick suggestions. But I have to go, we've already been here for almost 4 hours. While waiting for my discharge paperwork, a research assistant shows up to see if I'd be interested in participating in a study about I'm not sure what because my 3yr old is clambering to get out of that office. I agree anyway, get my discharge paperwork and fly out the door.

This summer, we were visiting family in Philadelphia and my son got sick. He was admitted to Children's Hospital of Philadelphia for a pulmonary exacerbation. Not a single morsel of information about him and his history with Cystic Fibrosis was available to the doctors at CHOP. Many phone calls were made between fellows from my personal cell phone to understand his medical history and doctors asked me the same questions day after day before we trusted and understood each other and hashed out what was going on in the days leading to our admission. I know that it was in Drew's best interest that we discuss everything about him ad nauseum, but it blew my mind that in this age of technology, there was no electronic medical health record that the doctors in Philadelphia could access to understand Drew to provide the best care for him quickly.

When Drew was a baby, we kept a diary of his formula intake and the corresponding output. We set up a nice little Excel chart and shared that with our dietician on a very regular basis. I credit that chart for his gained weight and reaching the 50th percentile by the time he was 6 months old and he has been able to maintain that to today when he is almost 3. I know that all patients aren't equal, but wouldn't it be great if that data, if our "patient reported outcomes" were cataloged somewhere so that we could share what we did and how it worked for us? Sure, I could take to the CF Mom's Facebook page and ask about what high calorie snacks work for their 3 year olds and hope that some of those moms are online and eager to provide some feedback. But once that question is asked and answered, it just simply vanishes into cyberspace. I cannot tell you how many times I've said, "I know I've seen that somewhere". Imagine the power of cataloging that data, those conversations, those findings. It can be used by patients, by doctors, by researchers and teachers. Just thinking about the power of that is what is driving me to stay involved and insist on nothing less.

Right now, much focus seems to be trying and often trying again to fix things that aren't working right - adherence reminders, transition projects, finding ways to ensure that patients don't feel isolated in this disease. Perhaps rather than just trying to change something that isn't working, we need to change the way we think about it and try something new.

The C3N for CF is a novel approach to healthcare. It changes the traditional doctor-patient relationship. The clinical staff are where they are because they chose to be. Patients don't choose their disease. It is something that happens to them and something that they have little control over. Patients want access to and control of their information because they want to be engaged and empowered regarding their care. Doctors should support this.

In order to overcome the current barriers, we need to identify the current barriers. In my opinion, clinicians seem puzzled as to why patients don't seem to want to engage in their own care and improving outcomes, but how many of you would be agreeable to regularly and reliably engaging in something that you don't like having, don't like doing, and don't want to spend any more time on than you already have to. We can't just assume that if we build it, they will come. We have to understand and engage patients as active participants in their disease management in a way that alleviates the burden already weighing heavily on them. I believe that the proposed interventions for the C3N for CF will do just that.

I've rewritten my story to reflect how I see the C3N changing things for the CF community. This may perhaps be an aspirational vision, but I don't think its unreasonable to expect to see these interventions causing a shift in the way people both think about and manage their disease.

It’s been 3 months since our last check in with the clinic. We've been tracking our data for the past 6 months and are interested to hear what, if any, trends they are seeing. Last week we got a summary of our data to review before our visit that helps us to identify any ongoing concerns that I've had since we were last seen. Our doctor has already reviewed this and is recommending that we meet with our RT to discuss alternative airway clearance techniques because she has seen that we have been checking that out on our message boards. Since she brought that up, I do agree that it could be useful to hear about some of our other options even though I haven't really thought about much about it since our last clinic visit. I also notice that Drew's interest in eating has declined considerably over the past 2 weeks. I let them know that I'd like to talk with the dietician to figure out what's going on. Drew's vitals (PFTs, O2, heart rate, blood pressure, etc), that we record with apps recommended by our clinic, have remained steady since our last visit. After returning our pre-clinic survey, our doctors review us along with the other patients scheduled to be seen during the upcoming week. We are given a green light by the clinical team and we decide together that we don't need to go into clinic for our appointment. I'm relieved because I would hate to pick something up when we're doing so well, and I wasn't sure if I was even going to be able to make it since my husband will be out of town for work and I haven't been able to find a babysitter.

During our appointment time, we meet via web conference. At the beginning of the call, the clinicians see that I have updated our medication list just prior to the appointment so we can skip that. They've also seen that we've been taking our prescribed medications pretty reliably and commend us for following the doctor’s orders. We look together at his behavioral data that we have been tracking and they point out that something is going on Tuesday and Thursdays. Per behavioral patterns and some survey responses from those days, he seems to have headaches and just doesn't feel great. After a few moments of conversation about what could be going on, we are able to identify that those are the days that he is in school and may not be getting as much to drink as he needs. We agree to try to keep him better hydrated at school and will continue to look at what happens.

The dietician and I talk for a few moments about his eating behaviors and she suggests that it may not be an appetite issue but rather a control issue. This is apparently common in preschoolers, and she asks the psychologist to hop on the call for a few minutes. The psychologist suggests a few techniques to try at mealtime and we make an appointment to check back in two weeks. Before she jumps off the call, I get a notification of our appointment through MyChart which I can easily add to my calendar. This is SO much easier than trying to talk to appointment scheduling before we leave or remembering to call them once we get home.

Our nurse hops on at the end to wrap up our appointment. She provides a document with some of the most common things parents of 3 year olds are discussing on our message boards. We can already swallow enzymes and don't ever have too much resistance getting him to do his treatments, but I had no idea that lizards and other reptiles carry pseudomonas! I wonder what else I don't know. I should check them out more often! She reminds us to drop our culture off at the lab and asks if we need any medication refills. We agree to continue doing what we're doing and will check back again in 3 months.

This summer, we were visiting family in Pennsylvania and my son got sick. He was admitted to the Children's Hospital there for a pulmonary exacerbation. I gave the doctors there access to his online personal health record, which includes all of the information from the team at our hospital as well as my own notes, behavioral data trends and conversations I've had with my clinicians about survey results and other. The doctors there were used to this because their patients also have this personal health record. After a phone call to my son’s doctor at our hospital, we understood what was going on in the days leading to our admission and had established a plan for treating the exacerbation. It felt good to know that there was an electronic medical health record that doctors everywhere were familiar with and could access to understand my son to provide the best care for him quickly.

It is a relief that Drew hasn't been seen in 3+ months and he is healthy. This new system is allowing me to really keep on top of how Drew is doing in an effortless way. I get insights into his behavior, health tips from a trusted source and I really feel more connected to the clinical team. I feel very in control and things seem to be going very well, so I'm happy to continue down this path if we will continue to see these kinds of results.

I think you're seeing my point, a point that was brought out in almost every session I sat through at the NACFC this year. The information that we currently have on patients in between visits is limited. Filling those gaps would give us a more complete picture of health. It would help doctors to intervene at appropriate times, times when patients might not call because in their mind "its just not that bad yet", but doctors are able to identify a problem or a pattern that lets them know the direction something that's "not that bad" is headed. They would be able to more easily determine if certain therapies were actually making a difference through the combine use of passive behavioral and active patient reported data, looking at a real-time view of what is going on with a patient, not what they remember to tell you when they are in clinic. The registry could be enhanced by identifying day to day trends and commonalities in patients sharing mutations. And all of this data can be used by researchers and scientists to figure out every last detail this disease and find therapies that work for every one of us. From a parent's perspective, the C3N is what we need to make life easier and improve outcomes while we wait for our cure.

Sunday, April 14, 2013

Our son Drew is 3 years old and he has Cystic Fibrosis, a life
shortening genetic disease for which there is no cure. This year, as we begin
our annual fundraising, we wanted to share with you two things that are driving
our efforts this year. One is a Cystic Fibrosis conference we attended a couple
weeks ago which I'll talk about below. The other is the wake that we recently
attended. Alicia, a local 20 year-old girl with Cystic Fibrosis, passed
away a few days ago. She had been very sick for a few years and was
literally on her death bed in February when she got the call that all of us who
knew her and her family had hoped for - new lungs became available for
her. Alicia was going to receive her life-saving double lung transplant.
It seemed that she was given a new lease on life. The surgery went reasonably
well, though things got a little dicey in the days immediately after.
Still, the day we left for our conference in D.C., I spoke with Alicia’s dad,
who said that Alicia was getting stronger every day. When we came back
from D.C., we found out that a couple days after we left, she got an infection
that she just couldn't fight. Her entire body had become so worn down from
living with Cystic Fibrosis for 20 years. Just when we thought that she had
found a way to beat CF, the disease won. When we saw Alicia’s father at her
funeral services and asked him how he was doing, he fought back his tears and
told us "I lost my baby girl". It was impossible for me to not
think about myself in that same position someday. As heartbreaking as Alicia's
passing is to us, we know that there is hope for a better future for
Drew.

A couple weeks ago, we spent some time in D.C.
and Virginia working with the Cystic Fibrosis Foundation. Our first day
there was spent on Capitol Hill meeting with congressmen and their staffers to
talk to them about the Foundation's policy agenda. We met with the
offices of 5 representatives and both senators from Ohio. It was
interesting and exciting, both to know that we were advocating for our cause
and because we were able to meet some other remarkable folks from the CF
community who share our passion for the cause. I’ve been working as the State
Advocacy Chair for a while now, and our
day on the hill convinced me of the importance of getting even more involved.

The second part of our trip was spent at the Cystic
Fibrosis Foundation’s Volunteer Leadership Conference. There were more
than 400 people there, about half volunteers and half foundation staff from around
the country. Simply: it was inspiring. Several things became very
clear to us:

·There is still much work to be done.
Currently 4% of the population benefits from a breakthrough new Cystic Fibrosis
drug, Kalydeco. Unfortunately, Drew's not in that group. As many as
another 91% of CFers could benefit from next generation advances. Drew
would be among those people, but it will take several years and hundreds of
millions of dollars, and success is not guaranteed.

·I can and must do more to find the cure for
Drew.

We
met some remarkable people at the conference who have made it their personal
mission to do everything in their power to help find a cure. We came away
with a lot of things to think about. Of course, most of us can do very
little to help with the science. Really, there are two things that we can
do: raise money and advocate. It is crucial work, the foundation relies
on it and we recognize that we have to do better. We truly appreciate
everyone that has donated and walked with us at Great Strides in the
past. In order to accomplish what we must accomplish, more is
needed. I'm sending you this letter because I want to ask you to join me,
to help me and more importantly to help Drew.

The
foundation is set to invest twice as much in drug research over the next three
years as they have in the last three - they're literally doubling down.
That means we need to double our results and that will require a bigger network
of donors. Money buys science and science buys more tomorrows for Drew.

This
is our impassioned plea. Our fundraising goal this year is an ambitious one,
but with the support of our family, friends, and all of you, hope that we will
be able to reach. We want to raise $30,000 this year for Team Doin’ It For
Drew. We are asking that you consider your giving capacity and help us in
whatever way you can.

Thank you for taking the
time to read about our life with Cystic Fibrosis. We truly appreciate any and
all support that you can offer.

Friday, April 5, 2013

When the CFF came to Cincinnati Children's at the end of January, I told a story about what some parts of our life are currently like living with Cystic Fibrosis and it highlighted fairly well the problems with the current system and how embracing a Collaborative Chronic Care network for CF would be highly beneficial. I must have said something right, because the CF Foundation is interested in what we're doing and is coming back with 7 members of their leadership team to learn more and hopefully partner with CCHMC on rolling this out. Here's what I have to tell them this time:I believe that there needs to be a defined difference between improving an existing process and creating a new one. Right now, much focus seems to be trying and often trying again to fix things that aren't working right - adherence reminders, transition projects, finding ways to ensure that patients don't feel isolated in this disease. Perhaps rather than just trying to change something that isn't working, we need to change the way we think about it and try something new.

The C3N for CF is a novel approach to healthcare. It changes the traditional doctor-patient relationship. The clinical staff are where they are because they chose to be. Patients don't choose their disease. It is something that happens to them and something that they have little control over. Patients want access to and control of their information because they want to be engaged and empowered regarding their care. Doctors should support this.

In order to overcome the current barriers, we need to identify the current barriers. In my opinion, clinicians seem puzzled as to why patients don't seem to want to engage in their own care and improving outcomes, but how many clinicians or just regular people do you know who would be agreeable to regularly and reliably engaging in something that you don't like having, don't like doing, and don't want to spend any more time on than you already have to. We can't just assume that if we build it, they will come. We have to understand and engage patients as active participants in their disease management in a way that alleviates the burden already weighing heavily on them. I believe that the proposed interventions for the C3N for CF will do just that.

I've rewritten my story to reflect how I see the C3N changing things for the CF community. This may perhaps be an aspirational vision, but I don't think its unreasonable to expect to see these interventions causing a shift in the way people both think about and manage their disease.

Its been 3 months since our last check in with the clinic. We've been tracking our data for the past 6 months and are interested to hear what, if any, trends they are seeing. Last week we got a summary of our data to review before our visit that helps us to identify any ongoing concerns that I've had since we were last seen. Our doctor has already reviewed this and is recommending that we meet with our RT to discuss alternative airway clearance techniques because she has seen that we have been checking that out on our message boards. Since she brought that up, I do agree that it could be useful to hear about some of our other options even though I haven't really thought about much about it since our last clinic visit. I also notice that Drew's interest in eating has declined considerably over the past 2 weeks. I let them know that I'd like to talk with the dietician to figure out what's going on. Drew's vitals (PFTs, O2, heart rate, blood pressure, etc), that we record with apps recommended by our clinic, have remained steady since our last visit. After returning our pre-clinic survey, our doctors review us along with the other patients scheduled to be seen during the upcoming week. We are given a green light by the clinical team and we decide together that we don't need to go into clinic for our appointment. I'm relieved because I would hate to pick something up when we're doing so well, and I wasn't sure if I was even going to be able to make it since my husband will be out of town for work and I haven't been able to find a babysitter.

During our appointment time, we meet via web conference. At the beginning of the call, the clinicians see that I have updated our medication list just prior to the appointment so we can skip that. They've also seen that we've been taking our prescribed medications pretty reliably and commend us for following the doctors orders. We look together at his behavioral data that we have been tracking and they point out that something is going on Tuesday and Thursdays. Per behavioral patterns and some survey responses from those days, he seems to have headaches and just doesn't feel great. After a few moments of conversation about what could be going on, we are able to identify that those are the days that he is in school and may not be getting as much to drink as he needs. We agree to try to keep him better hydrated at school and will continue to look at what happens.

The dietician and I talk for a few moments about his eating behaviors and she suggests that it may not be an appetite issue but rather a control issue. This is apparently common in preschoolers, and she asks the psychologist to hop on the call for a few minutes. The psychologist suggest a few techniques to try at mealtime and we make an appointment to check back in two weeks. Before she jumps off the call, I get a notification of our appointment through MyChart which I can easily add to my calendar. This is SO much easier than trying to talk to appointment scheduling before we leave or remembering to call them once we get home.

Our nurse hops on at the end to wrap up our appointment. She provides a document with some of the most common things parents of 3 year olds are discussing on our message boards. We can already swallow enzymes and don't ever have to much resistance getting him to do his treatments, but I had no idea that lizards and other reptiles carry pseudomonas! I wonder what else I don't know. I should check them out more often! She reminds us to drop our culture off at the lab and asks if we need any medication refills. We agree to continue doing what we're doing and will check back again in 3 months.

This summer, we were visiting family in Pennsylvania and my son got sick. He was admitted to the Children's Hospital there for a pulmonary exacerbation. I gave the doctors there access to his online personal health record, which includes all of the information from the team at our hospital as well as my own notes, behavioral data trends and conversations I've had with my clinicians about survey results and other. The doctors there were used to this because their patients also have this personal health record. After a phone call to my son’s doctor at our hospital, we understood what was going on in the days leading to our admission and had established a plan for treating the exacerbation. It felt good to know that there was an electronic medical health record that doctors everywhere were familiar with and could access to understand my son to provide the best care for him quickly.

It is a relief that Drew hasn't been seen in 3+ months and he is healthy. This new system is allowing me to really keep on top of how Drew is doing in an effortless way. I get insights into his behavior, health tips from a trusted source and I really feel more connected to the clinical team. I feel very in control and things seem to be going very well, so I'm happy to continue down this path if we will continue to see these kind of results.

I'll let you know what the Foundation thinks when they hear it in just a couple of weeks!

The long version:
Per my last post, Drew had a bronch last Friday and on Monday afternoon we found out that he grew achromobacter. We scheduled a PICC for Tuesday morning. Thank God my neighbor was on spring break and could watch the other kids all week because we needed her big time. I took him in at 9am for an 11am PICC placement. They only sedate kids for PICCs if a) they just can't handle it or b)they can be in patient for a recovery period. I usually insist on no sedation because I always want to go home. So they papoose him and a whole army of people use videos and toys to distract him while we restrain him for this sterile and somewhat dangerous procedure where they place an IV line into an artery in his arm that leads to his heart. Anyway, they found a good vein in his left arm and we got to work. The worst part is the lidocaine they use to numb the area because it burns and stings like a mother. He was so so brave and didn't cry or anything getting up onto the table, but you could tell that he was getting nervous when we started holding him down. Then the stick came and he just cried. He just looked at me and cried and cried and cried. They got the line in and then blew a vein. UGH! So they tried again. This time they got it in and started to feed it up his arm and boom, another vein blew. They told me they've never had this happen before. My poor baby was hurting big time, and now they had to switch to the other arm. It took almost an hour and a lot of sticks, but they got it in. We had a small amount of time to grab lunch and when we started to eat he cried to me that his "arms were broken". With lidocaine in both arms, he was totally numb and couldn't use his arms to get food to his mouth. He was so sad!

We headed over to the ER then to administer our first dose of antibiotics to make sure he didn't have a reaction. This is standard procedure. He was exhausted by this point and so was I, but a day in the hospital for 2 weeks at home was worth it. Each med takes 35-40 min to administer and we do 2 meds each 3 times a day. We are spending almost 4 hours a day now administering IV antibiotics from home. We. Are. Exhausted. I don't like to complain, I don't feel it gets you anywhere. But holy crap are we all tired. As I've said before, this whole CF thing is something that our whole family lives with. Mom and dad are tired, physically, mentally. emotionally, and the kids are all aware of that. There is fighting, acting up, throwing fits. The phone calls and coordination necessary to do this all from home requires a small business logistics department, and its a role that I'm having to fill. That means that when the phone rings I need to get it, even if I'm in the middle of a game, or if we're outside, or whatever we're doing. I hate when they're upset because of something I'm doing, namely not giving them the attention that they need from me. They're really being pretty good, given their ages and what I've been asking of them. But its hard.

To add to things, the Ginger.io gig I've been after since November finally came to fruition and training for that started this week. Oh yeah, and SEVEN members of the Cystic Fibrosis Foundation leadership team are coming back to CCHMC for a meeting on the C3N at the end of April so I've been working about 10 hours a week prepping for that. I am so excited for this meeting. I've been working on this for almost a year, trying to bring a C3N to Cystic Fibrosis, and it is about to go down. For real! But it's been busy around here and I'm ready for a weekend of going to bed early and waking up later. We will have that again in 2 weeks when the IV's are done because right now we have to run them from 11:30-1am and then again at 7am . Hopefully though it kills this bacteria and Drew is healthier and then we can implement this whole C3N for the CF community and improve things for everyone and then cure this disease and get on with our lives. Or something like that.

I was on a call today about N of 1 trials, an intervention that is a part of the C3N platform. It was a really good presentation and explained very nicely how this sort of thing would improve healthcare and outcomes for a person living with a chronic disease...like CF. All of these interventions and ideas that I have shared have been and are being piloted in the IBD community, and the Ginger.io work I'm doing will be with the GI clinics at CCHMC, but I see and I think I have been able to show the right people within the CF community the value that a concept like this could provide to us. We're getting there people, and I won't stop until we are. Watch this if you have a chance, it's good! http://vimeo.com/63423056

Monday, April 1, 2013

Last week I was invited to attend a tour of the CF Care and Research facilities at Cincinnati Children's, not as a guest, but as a parent who could offer real life experience to the other folks who were there and interested in investing either their time or money into the CF Foundation. I talked a lot about all that I do at Children's and within the CF community. I personally find very little benefit to sitting around and grumbling about how horrible things are and have the perspective that if you want something to change then go make it happen. One of the guys there asked how people stay positive and if there is support to help folks maintain a hopeful attitude, like the one that I had, when it seems to easy to get knocked down. I answered by saying that while there is great support from both doctors and other clinicians, social workers and psychologists, other parents who are in your shoes, a lot of it, I think, has to do with your own personal attitude and frankly I'm not sure whose supposed to be responsible for that. Sure, this disease sucks and takes a lot of time and energy, but if you don't fight for what you believe it then everything will remain the same and nothing will change. I try to find the positive, look toward the good, make things better.

A couple of months ago, after I attended the North American CF Conference, I wrote about one of the sessions I'd attended on Infection Control. I have, since the beginning of time [2010] felt that if Drew was culturing a new bacteria that he was getting it from the clinic. They always tell you that many of these bacteria are in the natural environment and while totally unharmful to non-CF kids, CF kids are just susceptible to getting them and that you'd make yourself nuts trying to figure out if it came from the bathtub or the swing set or the museum and that kids can't live in a bubble and yadda yadda yadda. I listened...mostly. Everyone who knows us knows that we are cautious but that we don't by any means live in a bubble. So when we go to the doctor perfectly healthy and it comes back that we're growing some funky new bacteria, I can't help but think that he got it there. Back to the conference. I attended this session on infection control and my worst nightmare was becoming a reality. Researchers had started using DNA in bacteria to genotype them and find out exactly where they were coming from and what they found was that patients who were in the clinic on the same days or who shared a hospital room one after another were sharing identical bacteria. Pardon my mouth, but MOTHER FUCKER!!! I KNEW IT!! Even with superb infection control at our clinic and in our hospital - great cleaning between patient visit, all clinicians gowning up before they come in, us wearing a mask and not touching anything from the time we leave the car until the time we get back into it - even with all of that in place, indirect transfer was happening. We all use the same elevator to get up to clinic. Joe Schmoe takes the elevator up to C5 at 9am and has some funk growing. He is coughing his whole way upstairs. We get in the same elevator an hour later for our appointment and end up with the same bacteria. Or a kid is reading a book while he's in for one of his 4 hour check up's and he sneezes. Later he closes the book and puts it away. The outside of the book gets wiped down, but not the pages. The next day my kid reads that same book and boom, we're contaminated. Do I know that's how its happening? No, not for sure. But I'm about to find out.

Later that afternoon, I tuned in to the Cystic Fibrosis Foundation's new Infection Control webcast. They pretty much presented exactly what I'd heard at NACFC (above). The new guidelines were focused on Foundation events and they said that the clinical recommendations would be out this summer, but basically they were saying that they have data that proves that patients are passing these harmful bacteria indirectly to one another by, for example, sharing an elevator even if not at the same time, touching handles or doorknobs, things like that. So their new guidelines say that only 1 CFer can be at any indoor foundation event and that at outdoor events CFers need to identify themselves and stay 6-10 feet away from other CFers. Guess whose taking this new evidence completely serious and will not be letting Drew anywhere near anything CF related anytime soon!

Later in the afternoon that day, I got a call from Drew's doctor about a strange bacteria he had grown in the culture we had done earlier in the week. He was growing something called Achromobacter, a very rare and not-normally-found-in-the-environment bacteria that is resistant to EVERYTHING. EVERYTHING!!! How did this happen? How did he get this?? Well, another kid that Drew's doctor sees also has/had achromobacter. Did he get it from him? Hard to say. But they're going to genotype it to find out. Achromobacter isn't the most horrible bacteria someone with CF can get, but its not great either because of its resistance to antibiotics. It also causes inflammation, and a kid with CF doesn't need any more inflammation in their aiways then they already have. Its sort of unknown what kind of long term damage achromobacter causes because its pretty rare, but it seems best to get rid of it if you can. One kind of rule of thumb with less common bacteria is to treat if its causing a problem. Drew's had this weird lingering cough for a couple of months that seemed to go away a little with antibiotics but kept coming back. So we decided that the best move would be a bronchoscopy to see what his airways looked like. If they looked clear like they have in the past, then we would do a culture and see what grew and make a plan from there. If they were junky, we would do IV's.

On Friday morning, we went in for our bronch. After being delayed an hour and a half, he finally had it done and he had a lot of gunk. We just got the culture results back today and he had achromobacter growing way down in his lower airways, so we decided that we needed to do some IV's. I learned something about antibiotic resistance today, that just because a bacteria is resistant to an antiobiotic in a petri dish doesn't always necessarily mean that it will be resistant in your body. Who knew! Anyway, tomorrow we're going in for a PICC and we're coming home. I didn't want to be in-patient and i'm fully capable of doing this all at home. We are doing to use two antibiotics, tobramycin and something else I can't remember and see where that gets us. After that treatment, we'll re-culture and make decisions from there. Hopefully, hopefully, it will go away. One step at a time.

When I talked to Drew's doc on the phone today, I expressed my fear of the clinic. The place that we need the most is looking like it might be the most dangerous place for us to be, and I'm terrified to take Drew back in there. She understands my fears and we're going to try to figure out a way to make this all still work. I totally get the importance of needing to see his doctors on a regular basis, but with something like Ginger.io and the C3N for CF (which I have a LOT to talk about that!) and us tracking our health on a regular basis, I don't know that we do need to go in that often. I am usually full of both good and bad ideas, and for this situation I've found myself in I cannot think of anything. I'm hoping that something will come along to either prove I'm right and provide a solution, or prove me wrong. I guess we shall see!

Looking back on the past couple of days and the question that the guy on the tour asked me, today I just wanted to cry. I wanted to scream and yell and punch CF in the face. Damnit to hell I hate this. Hate hate hate it. And I'm tired and I'm scared and I don't want Drew to have to go back to the hospital tomorrow for another procedure and then have to get this horrible medicine that will kill the bacteria but will also kill his appetite and make him not feel great and will prevent him from doing things like going to his first t-ball practice. I don't want to do this, no part of it! It's disheartening to think you're doing everything right and you still end up with a shitty situation. And while the next two weeks will be totally exhausting doing IV antibiotics every 8 hours around the clock on top of everything else that we have scheduled and planned and all the other crap that pops up n between, he will be better off in two weeks (God willing) than he is today and his health is the most important thing. So I take my moment to rant and curse the world, but tomorrow we will wake up and continue to do whatever we need to do to cure this bitch. Wanna donate? Click HERE!

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org